Literature DB >> 9450851

Plantar lipomatosis, unusual facial phenotype and developmental delay: a new MCA/MR syndrome.

M E Pierpont1, F J Stewart, R J Gorlin.   

Abstract

We describe two boys with global developmental delay and a phenotype of microcephaly, midface hypoplasia, enlarged fleshy ears, depressed nasal bridge, anteverted nostrils, central palatal ridge, and high forehead. Bilateral congenital fat pads are present anteromedial to the heels. Fetal finger and toe pads are present and palmar and plantar grooves are deeper than normal with "pillowing" of the areas between the grooves. No patients with similar clinical findings have been located, but these two children have a remarkably similar clinical presentation which we consider a "new" syndrome.

Entities:  

Mesh:

Year:  1998        PMID: 9450851

Source DB:  PubMed          Journal:  Am J Med Genet        ISSN: 0148-7299


  6 in total

1.  Pierpont syndrome: a collaborative study.

Authors:  Emma M M Burkitt Wright; Mohnish Suri; Susan M White; Nicole de Leeuw; Anneke T Vulto-van Silfhout; Fiona Stewart; Shane McKee; Sahar Mansour; Fiona C Connell; Maya Chopra; Edwin P Kirk; Koen Devriendt; Willie Reardon; Han Brunner; Dian Donnai
Journal:  Am J Med Genet A       Date:  2011-08-10       Impact factor: 2.802

2.  Expanding the phenotypic spectrum of ARID1B-mediated disorders and identification of altered cell-cycle dynamics due to ARID1B haploinsufficiency.

Authors:  Joe C H Sim; Susan M White; Elizabeth Fitzpatrick; Gabrielle R Wilson; Greta Gillies; Kate Pope; Hayley S Mountford; Pernille M Torring; Shane McKee; Anneke T Vulto-van Silfhout; Shalini N Jhangiani; Donna M Muzny; Richard J Leventer; Martin B Delatycki; David J Amor; Paul J Lockhart
Journal:  Orphanet J Rare Dis       Date:  2014-03-27       Impact factor: 4.123

3.  An animal model for Pierpont syndrome: a mouse bearing the Tbl1xr1Y446C/Y446C mutation.

Authors:  Yalan Hu; Peter Lauffer; Michelle Stewart; Gemma Codner; Steffen Mayerl; Heike Heuer; Lily Ng; Douglas Forrest; Paul van Trotsenburg; Aldo Jongejan; Eric Fliers; Raoul Hennekam; Anita Boelen
Journal:  Hum Mol Genet       Date:  2022-08-25       Impact factor: 5.121

4.  A specific mutation in TBL1XR1 causes Pierpont syndrome.

Authors:  Charlotte A Heinen; Aldo Jongejan; Peter J Watson; Bert Redeker; Anita Boelen; Olga Boudzovitch-Surovtseva; Francesca Forzano; Roel Hordijk; Richard Kelley; Ann H Olney; Mary Ella Pierpont; G Bradley Schaefer; Fiona Stewart; A S Paul van Trotsenburg; Eric Fliers; John W R Schwabe; Raoul C Hennekam
Journal:  J Med Genet       Date:  2016-01-14       Impact factor: 6.318

5.  Increased homozygosity in the first Hispanic patient with plantar lipomatosis, unusual facies, and developmental delay (Pierpont syndrome): a case report.

Authors:  Siobhán O'Keefe; Dieter T Wefuan; Jennifer B Humberson; Karen Schmidt; John Wiley
Journal:  J Med Case Rep       Date:  2016-08-12

6.  Pierpont syndrome-Report of a new patient.

Authors:  Vlora Ismaili-Jaha; Shqipe Spahiu-Konusha; Art Jaha
Journal:  Clin Case Rep       Date:  2021-02-18
  6 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.